Burkitt-like lymphoma with 11q aberration in a patient with AIDS and a patient without AIDS: Two cases reports and literature review.

OBJECTIVE: The aim of this study is to evaluate the clinicopathological features and the treatment of the Burkitt-like lymphoma with 11q aberration. METHODS: We reported two patients with Burkitt-like lymphoma with 11q aberration: a 56-year-old man with AIDS (case 1) and a 37-year-old woman (case 2) without AIDS. The biopsy of cervical lymph nodes showed Burkitt-like morphologic and immunophenotypic features. But both of them lack MYC rearrangement and carry an 11q-arm aberration with proximal gains and/or telomeric losses. The diagnosis was confirmed by pathological morphology, immunohistochemistry, and fluorescence in situ hybridization. RESULT: After a cycle of R-CTOEP (rituximab, cyclophosphamide, pirarubicin, vincristine, and prednisone) chemotherapy, case 1 refused to chemotherapy and radiotherapy and was followed up for 34 months without recurrence and new focus. Case 2 received R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) for two cycles and achieved PR (partial response). Then, the patient in case 2 received EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) for three cycles, and the right cervical mass disappeared. She achieved complete response and was followed up for 16 months without recurrence and new focus. CONCLUSION: Burkitt-like lymphoma with 11q abnormalities resembles Burkitt lymphoma morphologically but lacks MYC rearrangement and may have a better prognosis.

Application of 2016 WHO classification in the diagnosis of paediatric high-grade MYC-negative mature B-cell lymphoma with Burkitt-like morphological features.

AIMS: Historically, there has been no consensus on the diagnostic classification of high-grade B-cell lymphoma (HGBCL) with morphological features of Burkitt lymphoma (BL) but no MYC gene rearrangement (MYC-negative). The 2016 WHO classification of tumours of haematopoietic and lymphoid tissues has shed some light on this field with the modification of the grey-zone lymphoma with features intermediate between BL and diffuse large B-cell lymphoma, and the creation of several new entities. The aim of this study was to investigate how the revised WHO classification affects our practice in diagnosing these lymphomas in children. METHODS: We retrospectively reviewed cases of mature HGBCL diagnosed at our hospital between 2015 and 2018. RESULTS: Among 14 mature HGBCL cases with BL morphological features, 11 showed MYC rearrangement consistent with BL and 3 were MYC-negative. Two MYC-negative cases showed regions of 11q gain and loss by microarray consistent with Burkitt-like lymphoma with 11q aberration (BLL-11q). The third MYC-negative case showed diffuse and strong MUM1 expression, translocation involving 6p25 by chromosome analysis and IRF4 rearrangement by fluorescence in situ hybridisation analysis consistent with large B-cell lymphoma with IRF4 rearrangement (LBL-IRF4). All patients were treated according to applicable chemotherapeutic protocols and achieved remission. CONCLUSIONS: BLL-11q and LBL-IRF4, two newly defined entities, should be considered in paediatric MYC-negative mature HGBCL cases. Accurate diagnosis needs careful histopathological examination and proper cytogenetic testing. Since they have unique cytogenetic features, specific treatments for them may emerge in the future. Therefore, accurate diagnosis based on the 2016 WHO classification is clinically significant.

Burkitt-like lymphoma with 11q aberration.

Burkitt-like lymphoma with 11q aberration is a recently recognized diagnostic entity in the Revised 4th Edition of the World Health Organization (WHO) classification of lymphoid neoplasms. This diagnosis should be considered and cytogenetic array performed in patients with high-grade B-cell non-Hodgkin lymphomas without MYC rearrangement.

The 11q-Gain/Loss Aberration Occurs Recurrently in MYC-Negative Burkitt-like Lymphoma With 11q Aberration, as Well as MYC-Positive Burkitt Lymphoma and MYC-Positive High-Grade B-Cell Lymphoma, NOS.

OBJECTIVES: The latest revision of lymphoma's World Health Organization classification describes the new provisional entity "Burkitt-like lymphoma with 11q aberration" (BLL, 11q) as lacking MYC rearrangement, but harboring the specific11q-gain/loss aberration. We report genetic characteristics of 11 lymphoma cases with this aberration. METHODS: Classical cytogenetics, fluorescence in situ hybridization (FISH), and single nucleotide polymorphism/array comparative genomic hybridization. RESULTS: The 11q aberrations were described as duplication, inversion, and deletion. Array comparative genomic hybridization showed two types of duplication: bigger than 50 megabase pairs (Mbp) and smaller than 20 Mbp, which were associated with bulky tumor larger than 20 cm and amplification of the 11q23.3 region, including KMT2A. Six cases revealed a normal FISH status of MYC and were diagnosed as BLL,11q. Five cases showed MYC rearrangement and were diagnosed as Burkitt lymphoma (BL) or high-grade B-cell lymphoma, not otherwise specified (HGBL, NOS). CONCLUSIONS: The 11q-gain/loss is not specific for BLL, 11q, but occurs recurrently in MYC-positive BL and MYC-positive HGBL.